60 Childhood Behçet disease: clinical features, management and outcome of 13 cases

Abstract Background Behçet disease is a rare vasculitis that involves medium and small calibre arteries. Multisystem involvement is possible. Few are the papers that provide information about its clinical course, and outcome in the pediatric population. Also, the management is mainly based on adult guidelines. Objectives To shed light on the clinical course and outcome of juvenile Behçet disease. Methods A single center retrospective descriptive study of 13 children with Bechet disease. The mean follow-up period was for 3 years. Results We had 8 boys and 5 girls. Four of our patients were consanguineous. The mean age was 9 years. The mean interval between onset and diagnosis was 3 months. All of our patients had recurrent aphthous stomatitis with a median of 6 episodes per year. The other symptoms at diagnosis were concomitant genital ulcers in 4 cases, pseudo-folliculitis in 7 cases, urticarial rush in 4 cases, severe headache in 2 patients, stroke in one patient, prolonged and/or recurrent fever in 9 patients, uveitis in 4 cases, episcleritis in one case, arthritis in 5 cases, pulmonary hemorrhage in one case, and pyoderma gangrenosum in one case. The pathergy test was performed in all the patients, and was positive in 3 of them. Diagnosis was based on the International Criteria for Behcet's Disease. Acute phase reactants (ESR and CRP) were high in all patients. Meanwhile, granulocytosis was found in 10 patients. HLA 51 was positive in 8 patients, from whom three were siblings. All our patients were on steroids, five had colchicine, seven had azathioprine, two had methotrexate, two had cyclophosphamide, three had etanercept and one had tocilizumab. Complete remission was obtained in 8 cases in a mean time of 15 months, while five children had partial remission, and one boy lost sight of his left eye after a severe retinitis. Conclusion Behcet’s disease can cause severe sequalae if not diagnosed and treated on time. More studies are needed to establish management guidelines for pediatric population.


Background
Mycobacterium tuberculosis is an organism responsible for causing chronic granulomatous inflammation leading to active or latent tuberculosis (TB). Both pulmonary and extrapulmonary infections; including the skin, the eye, the cardiovascular, genitourinary and gastrointestinal systems can occur. Within the eye, it can cause global disease ranging from anterior or posterior uveitis to retinal TB. Within this abstract we demonstrate a case of chronic panuveitis with a diagnosis of ocular TB. Methodology Retrospective chart review Results We reviewed a 9-year-old-male in the rheumatology outpatient clinic who was on treatment for ocular TB with methotrexate 2.5 mg once a week, prednisone 5 mg once a day, maxitrol eye drops once a day and prednisolone eye drops twice a day. He presented with a red, painless left eye with deteriorating vision 2 years ago. Functional enquiry was unremarkable. He was noted to have a fixed dilated left pupil at 3-4mm but other examinations were normal. Extensive investigations were carried out, including an Interferon-gamma release assay (IGRA)-which was positive. Toxoplasma antibody test, Treponema pallidum Haemagglutination test, and chest X-ray were all negative. He was then started on the above-mentioned therapies with marked improvement. A decision on increasing the methotrexate dose if steroid tapering showed no improvement was undertaken and a plan on reassessing the patient with ANA, ACE and HLA B51 was made, to ensure complete workup. Discussion and conclusion Intra or extra ocular TB (OTB) manifestations may result as a direct inoculation of the organism in ocular fluid or as a systemic immune response initiated by an infection from a distant site. There is a myriad of ocular manifestations of OTB which make forming a clinical diagnostic criterion difficult. The gold standard for diagnosis is an ocular fluid culture showing organisms but the yield may be low and the risk of damage to surrounding structures may be high. PCR tests done on the sample may yield clinical information but non ocular tests, such as the IGRA and tuberculin tests may also be employed. Therapy depends on active vs latent disease and mainly involves antiinflammatory agents and anti-TBs. Ocular TB may lead to sudden and irreversible blindness with high socioeconomic implications and hence, an index of suspicion in areas with a high disease burden should be kept as diagnosis poses a challenge. Background Behç et's disease is a multisystem vasculitis whose pathogenesis remains unclear. Although usually described in young adults, it may begin in childhood. The diagnosis is clinical, based on international criteria. The limitations of early diagnosis are related to the progressive onset of symptoms and the variety of differential diagnoses at this age in the absence of a pathognomonic diagnostic test.

Objectives
To report the epidemiological features of our series and to compare the 2015 pediatric criteria with the 2014 and 1990 international criteria.

Materials and Methods
This is a monocentric, retrospective study of 31 children over an 11year period from January 2011 to December 2021. All patients suspected with Behcet's disease by a pediatric rheumatologist were included in the study, the 2014 international criteria ''ISG 2014'' being the gold standard classification criteria used in our center. Results 31 cases of Behcet's were collected. The mean age was 10 years (5.5-16) AE 2.87 years. A female predominance is noted with a sex ratio F/M of 1.21. A quarter of the patients were from a consanguineous marriage and 38.7% had a family history of Behcet disease. Mucocutaneous manifestations were represented by recurrent oral aphthosis 87.1%, genital aphthosis 29%, pseudofolliculitis 48.4%, erythema nodosum 6.5% and acne lesions 3.2%. The pathergy test was positive in 1 case. Ocular involvement was reported in 29% and joint involvement in 45.2%. Thromboembolic complication was seen in 9.6% and neurobehcet in one patient. The HLA B51 antigen was present in 45.2% of cases. In our series 38.7% respond the pediatric criteria, while 61.3% met the 2014 international criteria and 35.5% met the 1990 international criteria. The 2015 pediatric criteria have respectively a sensitivity and specificity of 63.2% and 100% (p ¼ 0.002) compared with the 2014 international criteria taken as gold standard, as well as the 1990 international criteria with a respective sensitivity and specificity of 52.6% and 91.7% (p ¼ 0.034).

Conclusion
Our study highlights a female predominance, a high rate of consanguinity and familial Behcet. The sensitivity and specificity of the 1990 pediatric and international criteria appear to be better than those of 2014, with a more significant trend for the 2015 pediatric criteria (p ¼ 0.002).

Background
Behç et's disease is a peculiar vasculitis that affects both small and large vessels of the arterial and venous circulations.

Objectives
We present the case of two school-aged boys who presented with acute cerebral venous thrombosis, leading to the diagnosis of Behç et's disease. Case presentation -An 8-year-old boy is admitted for severe headaches with nausea. The clinical examination notes a febrile child with marked aphtosis (with a history of genital ''burns''). ESR and CRP are slightly elevated, while cerebral CT angiography reveals superior sagittal sinus occlusion. Angio-MRI confirms thrombophlebitis.
-Another 9-year-old boy is referred for abdominal and spinal pain with intense headaches; accompanied with a subacute fever for >15 days. CT angiography revealed (reveals) stenosis of the longitudinal sinuses. For both children, the pathergy test, skin biopsy, and ophthalmic examination completed the whole picture of the Behç et disease.

Discussion
Major vascular manifestations of Behç et's disease include venous and/or arterial occlusion and aneurysm. Cerebral venous thrombosis (CVT) is very particular and might lead to an intracranial hyper-pressure, as in our cases. Steroids remain the initial reference treatment, with particular attention to anticoagulation and the addition/relay with colchicine. Conclusion Cerebral venous thrombosis may reveal Behç et's disease, especially in the pediatric population. The prognosis is poor and requires a rigorous, multidisciplinary follow-up.

Background
Behç et disease is a rare vasculitis that involves medium and small calibre arteries. Multisystem involvement is possible. Few are the papers that provide information about its clinical course, and outcome in the pediatric population. Also, the management is mainly based on adult guidelines. Objectives To shed light on the clinical course and outcome of juvenile Behç et disease.

Methods
A single center retrospective descriptive study of 13 children with Bechet disease. The mean follow-up period was for 3 years.

Results
We had 8 boys and 5 girls. Four of our patients were consanguineous. The mean age was 9 years. The mean interval between onset and diagnosis was 3 months. All of our patients had recurrent aphthous stomatitis with a median of 6 episodes per year. The other symptoms at diagnosis were concomitant genital ulcers in 4 cases, pseudofolliculitis in 7 cases, urticarial rush in 4 cases, severe headache in 2 patients, stroke in one patient, prolonged and/or recurrent fever in 9 patients, uveitis in 4 cases, episcleritis in one case, arthritis in 5 cases, pulmonary hemorrhage in one case, and pyoderma gangrenosum in one case. The pathergy test was performed in all the patients, and was positive in 3 of them. Diagnosis was based on the International Criteria for Behcet's Disease. Acute phase reactants (ESR and CRP) were high in all patients. Meanwhile, granulocytosis was found in 10 patients. HLA 51 was positive in 8 patients, from whom three were siblings. All our patients were on steroids, five had colchicine, seven had azathioprine, two had methotrexate, two had cyclophosphamide, three had etanercept and one had tocilizumab. Complete remission was obtained in 8 cases in a mean time of 15 months, while five children had partial remission, and one boy lost sight of his left eye after a severe retinitis. Background Behç et's disease (BD) is a chronic inflammatory disease characterized by recurrent oral aphthous and genital ulcers accompanied by eye, joint, skin, gastrointestinal and central nervous system involvement. The vascular involvement may affect both the arterial and venous systems. Nailfold videocapillaroscopy (NVC) is an easy and non-invasive method used in the evaluation of microcirculation. With this study, we aimed to find the characteristics and prevalence of nailfold capillary alterations in